Increased frequency of interleukin-4 and reduced frequency of interferon-γ and IL-17-producing CD4+ and CD8+ cells in scleromyxedema.
J Eur Acad Dermatol Venereol
; 34(5): 1092-1097, 2020 May.
Article
en En
| MEDLINE
| ID: mdl-31912592
ABSTRACT
BACKGROUND:
Little is known about the pathogenesis of scleromyxedema, a life-threatening fibromucinosis disease with immunological dysregulation.OBJECTIVES:
To investigate on T-cell phenotype, function and cytokine biology in search of new insights supporting the immunopathogenesis of the disease.METHODS:
We analysed the frequency of circulating lymphocyte subsets, the T-cell maturation stage, the generation of antigen-specific T-cell lines and T-cell cytokine secretion.RESULTS:
The analysis of T-cell maturation stage and the TCR spectratyping findings revealed that scleromyxedema patients showed clear immunological signs of long-lasting immune system activation and stimulation leading to a skewed T-cell repertoire. Moreover, these analyses showed that both CD4+ and CD8+ T cells from scleromyxedema patients have a profound deficiency (even after stimulation) relatively to the production of IFN-γ and IL17 with respect to healthy donor control cells, while they are massively skewed towards IL4 secretion after stimulation.CONCLUSIONS:
Our data indicate that a chronic Th2-skewed T-cell response against an unknown target antigen leading to abnormally high IL4 secretion, a pro-fibrotic cytokine, is a main immunological hallmark of scleromyxedema patients. These results, never reported before, may have a translational therapeutic value due to the availability of anti-IL4 agents such as dupilumab.
Texto completo:
1
Colección:
01-internacional
Base de datos:
MEDLINE
Asunto principal:
Interleucina-4
/
Escleromixedema
Límite:
Humans
Idioma:
En
Revista:
J Eur Acad Dermatol Venereol
Asunto de la revista:
DERMATOLOGIA
/
DOENCAS SEXUALMENTE TRANSMISSIVEIS
Año:
2020
Tipo del documento:
Article
País de afiliación:
Italia